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Systemic-to-pulmonary blood supply in tetralogy of Fallot with pulmonary atresia

Published online by Cambridge University Press:  30 July 2009

Renata N. Rossi ,
Alison Hislop ,
Robert H. Anderson ,
F. Maymone Martins  and
Andrew C. Cook
Renata N. Rossi
Affiliation:
Paediatric Cardiac Unit, Hospital de Santa Cruz, Lisbon, Portugal
Alison Hislop
Affiliation:
Vascular Biology Unit, Institute of Child Health, London, UK
Robert H. Anderson
Affiliation:
Cardiac Unit, Institute of Child Health, London, UK
F. Maymone Martins
Affiliation:
Paediatric Cardiac Unit, Hospital de Santa Cruz, Lisbon, Portugal
Andrew C. Cook*
Affiliation:
Cardiac Unit, Institute of Child Health, London, UK
*
Cardiac Unic, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. Tel: +44 207 905 2343; Fax: +44 207 905 2324; E-mail: [email protected]
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Abstract

Tetralogy of Fallot with pulmonary atresia is one of the most challenging congenital cardiac malformations, for the morphologist, cardiologist and surgeon alike. Much of the difficulty in this lesion concerns the nature and development of pulmonary arterial supply, and the manner in which complete segmental supply to the lungs can be successfully restored or maintained. In this review, we discuss the anatomy and nomenclature of the lesion, emphasising the variability that can occur in pulmonary arterial anatomy, particularly in the presence of systemic-to-pulmonary collateral arteries. We speculate on the likely embryologic origins of these connections. Then by means of anatomic-clinical correlations, we emphasise the diagnostic approach to delineating the origin and extent of the pulmonary vasculature.

Keywords

Pulmonary atresia with ventricular septal defectdevelopmentbronchial arteriesangiography
Type
Continuing Medical Education
Information
Cardiology in the Young , Volume 12 , Issue 4 , July 2002 , pp. 373 - 388
Copyright
Copyright © Cambridge University Press 2002

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References

1.Baker, E. Tetralogy of Fallot with pulmonary atresia. In: Anderson, RH, Baker, EJ, Macartney, FJ, Shinebourne, EA, Rigby, ML, Tynan, M (eds). Paediatric Cardiology, (2nd edn) Vol. 2. Churchill Livingstone, Edinburgh, 2002, pp 1251–1279.Google Scholar
2.Anderson, RH, Seo, JW, Ho, SY. The pulmonary arterial supply in tetralogy of Fallot with pulmonary atresia. In: Yacoub, M, Pepper, J (eds). Annual of Cardiac Surgery 1990–91. Current Science Ltd, London, 1991, pp 77–83.Google Scholar
3.Anderson, RH, Devine, WA, Del Nido, P.The surgical anatomy of tetralogy of Fallot with pulmonary atresia rather than pulmonary stenosis. J Cardiac Surg 1991; 6: 41–59.CrossRefGoogle ScholarPubMed
4.Huntington, GS.The morphology of the pulmonary artery in the mammalia. Anat Rec 1919, Vol. 17.CrossRefGoogle Scholar
5.Congdon, ED.Transformation of the aortic arch system during the development of the human embryo. Carnegie Inst Contr Embryol 1922; 14:47–110.Google Scholar
6.Boyden, EA.The time lag in the development of bronchial arteries. Anat Rec 1970; 166: 611–614.CrossRefGoogle ScholarPubMed
7.Hall, SM, Hislop, AA, Pierce, CM, Haworth, S.Prenatal origins of human intrapulmonary arteries formation and smooth muscle maturation. Am J Respir Cell Mol Biol 2000; 23: 194–203.CrossRefGoogle ScholarPubMed
8.DeMello, D, Sawyer, D, Galvin, N, Reid, ML.Early fetal development of the lung vasculature. Am J Respir Cell Mol Biol 1997; 16: 568–581.CrossRefGoogle ScholarPubMed
9.DeRuiter, MC, Gittenberger-de, Groot, Poelmann, RE, Vanlperen, L, Mentink, MM.Development of the pharyngeal arch system related to the pulmonary and bronchial vessels in the avian embryo. With a concept on systemic-to-pulmonary collateral artery formation. Circulation 1993; 87: 1306–1319.CrossRefGoogle Scholar
10.Boyden, EA.The developing bronchial arteries in a fetus of twelfth week. Am J Anat 1970; 129: 357–368.CrossRefGoogle Scholar
11.Deffebach, ME, Charan, NB, Lakshminarayan, S, Butler, J.The bronchial circulation. Small, but a vital attribute of the lung. Am Rev Respir Dis 1987; 135: 463–481.Google ScholarPubMed
12.Wagenvoort, CA, Wagervoort, N.Arterial anastomoses, bronchopulmonary arteries, and pulmobronchial arteries in perinatal lungs. Lab Invest 1967; 16: 13–24.Google ScholarPubMed
13.O'Rahilly, R, Debson, H, King, TS.Subclavean origin of bronchial arteries. Anat Rec 1950; 108: 227–238.CrossRefGoogle Scholar
14.Haworth, SG, Macartney, F.Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Br Heart J 1980; 44: 14–24.CrossRefGoogle ScholarPubMed
15.Ho, SY, Catani, G, Seo, JW.Arterial supply to the lungs in tetralogy of Fallot with pulmonary atresia or critical pulmonary stenosis. Cardiol Young 1992; 2: 65–72.Google Scholar
16.Liao, PK, Edwards, WD, Julsrud, PR, Puga, FJ, Danielson, GK, Feldt, RH.Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect. J Am Coll Card 1985; 6: 1343–1350.CrossRefGoogle ScholarPubMed
17.De Ruiter, MC, Gittenberger-De Groot, AC, Borges, AJ, Elzenga, NJ.The restricted surgical relevance of morphologic criteria to classify systemic-to-pulmonary collateral arteries in pulmonary atresia with ventricular septal defect. J Thorac Cardiovasc Surg 1994; 108: 692–699.CrossRefGoogle Scholar
18.Schulze-Neick, I, Ho, Y, Bush, A, Rosenthal, M, Franklin, R, Redington, A, Penny, J.Severe airflow limitation after the unifocalization procedure. Clinical and morphological correlates. Circulation 2000; 102 (Suppl III): III–142—III–147.CrossRefGoogle ScholarPubMed
19.Negus, VE, Appleton, AB, Brock, RC, Foster-Carter, AF.The nomenclature of broncho-pulmonary anatomy. An international nomenclature accepted by the Thoracic Society. Thorax 1950; 5: 222–228.Google Scholar
20.Boyden, EA.A critique of the international nomenclature of bronchopulmonary segments. Chest 1953; 23: 226–229.Google ScholarPubMed
21.Shimazaki, Y, Maehara, T, Blackstone, EH, Kirklin, JW, Bargeron, LM.The structure of the pulmonary circulation in tetralogy of Fallot with pulmonary atresia. A quantitative cineangiography study. J Thorac Cardiovasc Surg 1988; 95: 1048–1058.CrossRefGoogle ScholarPubMed
22.Anderson, RH, Thiene, G.Categorization and description of hearts with a common arterial trunk. Eur J Cardiovasc Surg 1989; 3: 481–487.CrossRefGoogle ScholarPubMed
23.Gerlis, LM, Ho, SY, Anderson, RH, Da, Costa. Persistent 5th aortic arch: a greater pretender: three new covert cases. Br Heart J 1989; 52:440–445.CrossRefGoogle Scholar
24.Pahl, E, Fong, L, Anderson, RH, Park, SC, Zuberbuhler, JR.Fistulous communications between a solitary coronary artery and the pulmonary arteries as the primary source of pulmonary blood supply in tetralogy of Fallot with pulmonary valve atresia. Am J Card 1989; 63: 140–143.CrossRefGoogle ScholarPubMed
25.Thiene, G, Frescura, C, Bortolotti, U, Del Maschio, A, Valente, M.The systemic pulmonary circulation in pulmonary atresia with ventricular septal defect: concept of reciprocal development of the fourth and sixth aortic arches. Am Heart J 1981; 101: 339–344.CrossRefGoogle ScholarPubMed
26.Frescura, C, Talenti, E, Pellegrino, PA, Mazzucco, A, Faggian, G, Thiene, G.Coexistence of ductal and systemic pulmonary arterial supply in pulmonary atresia with ventricular septal defect. Am J Cardiol 1984; 15: 348–389.CrossRefGoogle Scholar
27.Waldman, JD, Karp, RB, Gittenberg-de Groot, AC, Agarwala, B, Glasgow, S.Spontaneous acquisition of discontinuous pulmonary arteries. Ann Thorac Surg 1996; 62: 161–168.CrossRefGoogle ScholarPubMed
28.Thiene, G, Frescura, C, Bini, R, Valente, M, Gallucci, V.Histology of pulmonary arterial supply in atresia with ventricular septal defect. Circulation 1979; 60: 1066–1074.CrossRefGoogle ScholarPubMed
29.Haworth, SG.Collateral arteries in pulmonary atresia with ventricular septal defect: a precarious blood supply. Br Heart J 1980; 44: 5–13.CrossRefGoogle ScholarPubMed
30.Haworth, SG, Rees, PG, Taylor, JFN, Macartney, J, De Leval, M, Stark, J.Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Effect of systemic pulmonary anastomosis. Br Heart J 1981; 45: 133–141.CrossRefGoogle ScholarPubMed
31.Kirklin, JW, Barratt-Boyes, BG. Ventricular septal defect and pulmonary stenosis or atresia. In: Kirklin, JW, Barratt-Boyes, BG (eds). Cardiac surgery (2nd edn) Vol. 2. New York: Churchill Livingstone, 1992, pp 861–1012.Google Scholar
32.Faller, K, Haworth, SG, Taylor, JFN, Macartney, FJ.Duplicate sources of pulmonary blood supply in pulmonary atresia with ventricular septal defect. Br Heart J 1981; 46: 263–268.CrossRefGoogle ScholarPubMed
33.Freedom, RM, Mawson, JB, Yoo, SJ, Benson, LN. The pulmonary circulation in pulmonary atresia. In: Freedom, RM (ed.). Congenital heart disease: textbook of angiocardiography, Vol. 1. New York: Futura Publishing Co, 1997, pp 535–595.Google Scholar
34.McGoon, MD, Fultum, RE, Davis, GD, Ritter, DG, Neill, CA, White, RJ.Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect. Circulation 1977; 56: 473–479.CrossRefGoogle ScholarPubMed
35.Tchervenkov, CI, Salasidis, G, Cecere, R et al. , One-stage midline unifocalization and complete repair in infancy versus multiplestage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals. J Thorac Cardiovasc Surg 1997; 114:727–737.CrossRefGoogle ScholarPubMed
36.Luciani, GB, Wells, WJ, Khong, A, Starns, VA.The clamshell incision for bilateral pulmonary artery reconstruction in tetralogy of Fallot with pulmonary atresia. J Thorac Cardiovasc Surg 1997; 113:443–452.CrossRefGoogle ScholarPubMed
37.Reddy, VM, McElhinney, DB, Amim, Z et al. , Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation 2000; 101: 1826–1832.CrossRefGoogle ScholarPubMed
38.Lofland, GK.The management of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries by definitive single stage repair in early infancy. Eur J Cardiothorac Surg 2000; 18: 480–486.CrossRefGoogle ScholarPubMed
39.Acherman, JR, Smallhorn, JF, Freedom, RM.Echocardiographic assessment of pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect. J Am Coll Cardiol 1996; 28: 1308–1313.CrossRefGoogle ScholarPubMed
40.Carvalho, JS, Silva, CM, Rigby, ML, Shinebourne, EA.Angiographic diagnosis of anomalous coronary artery in tetralogy of Fallot. Br Heart J 1993; 70: 75–78.CrossRefGoogle ScholarPubMed
41.Gersony, WM.Obstruction to pulmonary venous return obscured by decreased pulmonary blood flow. Chest 1973; 64: 283.CrossRefGoogle ScholarPubMed
42.Carrotti, A, Di Donato, RM, Squitieri, C, Guccione, P, Catena, G.Total repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: an integrated approach. J Thorac Cardiovasc Surg 1998; 116: 914–923.CrossRefGoogle Scholar
43.Momma, K, Kondo, C, Matsouka, R.Tetralogy of Fallot with pulmonary atresia associated with chromosome 22q11 deletion. J Am Coll Cardiol 1996; 27: 198–202.CrossRefGoogle ScholarPubMed
44.Chessa, M, Butera, G, Bonhoffer, P et al. , Relation of genotype 22q11 deletion to phenotype of pulmonary vessels in tetralogy of Fallot and pulmonary atresia-ventricular septal defect. Heart 1998; 79: 186–190.CrossRefGoogle ScholarPubMed
45.Hofbeck, M, Rauch, A, Buheitel, G et al. , Monosomy 22q11 in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Heart 1998; 79: 180–185.CrossRefGoogle ScholarPubMed
46.Digilio, MC, Marino, B, Grazioli, S, Agostino, D, Giannotti, A, Dallapiccola, B.Comparison of occurrence of genetic syndromes in ventricular septal defect with pulmonic stenosis (classic tetralogy of Fallot) versus ventricular septal defect with pulmonary atresia. Am J Cardiol 1996; 77: 1375–1376.CrossRefGoogle Scholar
47.Anaclerio, S, Marino, B, Digilio, MC et al. , Pulmonary atresia with ventricular septal defect: prevalence of deletion 22q11 in the different anatomic patterns. Ital Heart J 2001; 2: 384–387.Google ScholarPubMed
48.Somerville, J, Saravalli, O, Ross, D.Complex pulmonary atresia with congenital systemic collaterals. Classification and management. Arch Mal Coeur Vaiss 1978; 71: 322–328.Google ScholarPubMed